Table 3.

Paraneoplastic and Other Autoimmune Neurological Disorders Organized by Anatomic Level.^a

Neuraxis Level	Disorder	Examples of Salient Antibodies
Eye	Autoimmune retinopathy, optic neuropathy	CRMP-5-IgG, NMO-IgG
Cerebral cortex	Limbic encephalitis, encephalitides presenting primarily with seizures or cognitive dysfunction, myoclonus	ANNA-1, VGKC complex Ab, NMDA receptor Ab
Diencephalon	Hypothalamic dysfunction, sleep disorders	Ma1/Ma2 Ab
Basal ganglia	Chorea, dystonia	CRMP-5 IgG
Cerebellum	Cerebellar ataxia/degeneration	PCA-1, P/Q type calcium channel Ab
Brain stem	Brain stem encephalitis, jaw dystonia Stiff-man phenomena (including stiffness, spasms, exaggerated startle, if widespread referred to as progressive encephalomyelitis with rigidity and myoclonus [PERM]), opsoclonus–myoclonus syndrome	ANNA-2, Ma1/Ma2, glycine receptor ab
Cranial nerves	Special senses, bulbar, motor neuropathies	ANNA-1, CRMP-5 IgG
Spinal cord	Myelopathy, myoclonus Stiff-man phenomena	CRMP-5 IgG, NMO-IgG, GAD65 Ab
Peripheral somatic nerves and ganglia	Sensory neuronopathy, sensorimotor neuropathies	ANNA-1, VGKC complex Ab
Neuromuscular junction	Lambert-Eaton syndrome, Myasthenia gravis	P/Q-type calcium channel Ab, muscle AChR Ab
Muscle	Polymyositis dermatomyositis, necrotizing myopathy	Jo1 Ab, SRP-54 Ab
Autonomic and enteric nervous system	Dysautonomias (pandysautonomia or limited dysautonomia, which includes gastrointestinal dysmotilities)	ANNA-1, alpha-3 ganglionic AChR

Abbreviations: AchR, acetylcholine receptor; ANNA, antineuronal nuclear antibody; CRMP5, collapsin-response mediator protein 5; PCA-1, purkinje cell cytoplasmic antibody type 1; VGKC, voltage-gated potassium channel; NMDA, N-methyl-d-aspartate; GABA-B, gamma-aminobutyric acid-B; Jo-1 Abs, anti-histidyl-tRNA synthetase Abs; NMO, neuromyelitis optica; IgG, immunoglobulin G; PERM=progressive encephalomyelitis with rigidity and myoclonus; SRP=signal recognition particle.

^aMany of these disorders can be multifocal.